<C1BEBCB320B1E8C5C2C7F62E687770>

CASE REPORTS pissn 1976-3573 eissn 2288-0941 THE KOREAN JOURNAL OF PANCREAS AND BILIARY TRACT 정상윤 1, 김효정 1, 김재선 1, 김백희 2, 이연호 1, 유양재 1, 정용 1, 박영태 1 1 고려대학교의과대학구로병원내과학교실, 2 병리학교실 Intraductal Papillary Neoplasm inducing the Intrahepatic Bile Duct Dilatation mimiking as Liver Abscess Sang Yoon Chung 1, Hyo Jung Kim 1, Jae Seon Kim 1, Baek hui Kim 2, Yun Ho Lee 1, Yang Jae Yoo 1, Yong Jeong 1, Young Tae Bak 1 1 Department of Gastroenterology and 2 Pathology, Guro Hospital, Korea University College of Medicine, Seoul, Korea Intraductal papillary neoplasm (IPN) of bile duct presents the tumor of a papillary surface comprising innumerable frondlike infolding of proliferation of columnar epithelial cells surroudings slender fibrovascular stalks. Neoplasm in the bile ducts which are dilated due to obstruction by a tumor itself, sloughed tumor debris, and excessive mucin. There are some researches of IPN of bile duct because they have same embryonic origin between IPN of bile duct and intraductal papillary mucinous neoplasm (IPMN) of pancreas. Patient with fever was suspected liver abscess in abdominal ultrasonography. Intraductal papillary neoplasm (IPN) of bile duct was suspected in additional imaging study. We experienced intraductal papillary neoplasm with high grade intraepithelial neoplasia after surgical resection. Here we report a case with the review of the reported literature. Key words: intraductal papillary neoplasm, liver, abscess 서론 1) 담관암의전암병변으로는담관상피내종양 (biliary intraepithelial neoplasia), 관내유두종양 (intraductal papillary neoplasm, IPN), 점액분비낭성종양 (mucinous cystic neoplasm), 선종등이있다. 1 이중담관의관내유두종양 (intraductal papillary neoplasm of bile duct) 은담관내로다발성으로유두상성장을하며, 종양자체나종양의잔해 (debri) 혹은과도하게분비되는점액에의한 Corresponding author. 김재선고려대학교의과대학구로병원소화기내과 152-703, 서울시구로구구로2동 97 Tel: 02-2626-3002 Fax: 051-254-3127 E-mail: kimjs@kumc.or.kr 담관폐쇄, 이로인한담관확장이다발성으로나타날수있는종양으로, 2,4 췌관내유두점액종양 (intraductal papillary mucinous neoplasm) 과발생병리학적으로유사한점이많다. 3 발열을주소로내원한환자가복부초음파에서간농양이의심되어, 간농양의원인을찾고자시행한추가영상검사에서담관내유두종이의심되었고, 수술후, 고도의이형성을동반한점액과분비간내담관내유두종양으로진단되었던 1예를경험하였기에문헌고찰과함께보고한다. 증례 평소건강하던 68세남자가내원전날부터발열, 오한, 상복부통증등을주소로내원하였다. 1년전고혈압을진단받아투약중이었으나, 그외특이병력은없었다. 34

35 Fig. 1. (A) Abdominal ultrasonography shows 8.3x7.6 cm sized multiseptated abscess in right posterior segment of liver. (S6). (B) Disproportional dilatation of bile duct with suspected mucosal nodularity in liver S6. Fig. 2. (A) ERCP shows filling defect in common bile duct, contrast media does not fill the common hepatic duct due to mucin. (B) Esophagogastroduodenoscopy shows mucin is secreted from major duodenal papilla.. Mucin and bile extracted by the basket Esophagogastroduodenoscopy 내원시체온 40, 혈압 100/50 mmhg, 맥박수 128 회, 호흡수 20 회였다. 우상복부의촉진에서경도의압통을동반하였으나, 종괴는촉지되지않았다. 말초혈액검사에서백혈구 11,600 /μl, 혈색소 11.4 g/dl, 혈소판 168,000 /μl 였고, CRP는 97.6 mg/dl이었다. 혈청생화 학검사에서 AST 33 IU/L, ALT 23 IU/L, 알칼리포스파타제 228 IU/L, 총빌리루빈 0.76 mg/dl, 아밀라제 71 U/L, CEA 7.4 ng/ml, CA 19-9 162.8 U/mL, HBsAg 음성, HBsAb 양성이었다. 고열의원인감별을위해시행한복부초음파검사에서

정상윤외 7 명 36 간우후엽에약 8.3 x 7.6 cm 크기의여러격벽으로나뉜농양성종괴가보였고, 이와연결된간내담관이간문부까지확장된소견을보였으며, 총담관역시 1.3 cm 으로확장되어있었다. (Fig. 1A) 간농양과담관확장의원인을담관의담석으로우선생각하고, 확인하기위해시행한복부자기공명췌담관조영술 (magnetic resonance cholangiopancreatography, MRCP) 에서췌관은정상이었고, 총담관의폐쇄소견은없었으나, 초음파에서관찰되었던간의낭성종괴와연결된우측간내담관이비대칭적인현저한확장을보여, 담관내점액분비종양이의심되었다. (Fig. 1B) 간우후엽의농성종괴에대해서간농양또는확장된담도내세균감염의의심하에광범위항생제투여와함께경피경간담즙배액술 (percutaneous transhepatic bile duct drainage) 을시행하였으며, 초기에 10 ml 정도의혼탁한농이배출된이후에는초록색의담즙만이지속적으로배액되었다. 이후발열및오한, 복통등은호전되었고, 증상이호전된후시행한내시경역행담췌관조영술 (endoscopic retrograde cholangiopancreatography, ERCP) 에서는주유두는발적소견이보였고, 총담관내에조영제가균일하게차지않는음영결손이의심되었으며, 간내담관내로도조영제의주입은어려웠다. (Fig. 2A). 내시경괄약근절개술후총담관내음영결손은담즙으로싸인많은양의끈적한젤리같은점액이바스켓으로배출되었다. (Fig. 2B) 점액과분비를일으키는담관내유두종양의심하에정확한진단및치료를위해우측간엽절제술을시행하였다. 수술후절제된조직의육안소견에서전체적으로 9 x 8 x 5 cm 크기의점액으로가득찬심한담도확장소견을보였으며이는튜브모양으로심하게확장된담도에연이어 5.5 x 5 x 2.4 cm 크기의다격벽낭성종괴모양을하고있었다. 현미경적병리소견에서담도상피는유두상증식을하고고도의상피내암을동반하여간내담관내유두종양으로진단되었다. (Fig. 3, Fig. 4) 이후환자는수술후특별한합병증없이호전되어퇴원하였다. 고찰 담관내유두종양은미세한섬유혈관중심의비정형담도상피세포의유두상성장을특징으로한다. 이중에서점액을분비하는경우가있어, 이를췌장의 IPMN과유사하게담관내유두상점액종양 (intraductal papillary mucinous tumor of the bile ducts) 4 으로분류되기도하였으나, 최근 WHO classification 에서는담관의관내유두종양으로분류하여, 고도의이형성과저도및중등도로분류하고있다. 1 췌장과담관은그발생학적기원이같은것으로알려져있어, 담관의관내유두종양은췌장에서발생하는췌관내점액유두종양과유사한점이많지만, 임상적으로서로다른특징을보이기도한다. 담관의유두종양은, 췌장의점액유두종양에비해여성에서흔하고, 혈청빌리루빈과알칼리포스파타제상승이더잘나타나고, CEA, CA 19-9 의상승이두드러진다. 이는다량의점액을분비하는경우담즙의흐름을방해하여담도의확장과폐쇄가동반되기때문인것으로생각되며 4,5 담석발생의가능성도보고되어있다. 6 이번증례에서도알칼리포스파타제, CA 19-9의높은상승을보였다. 또한담관내유두종양은악성화양상을보이는비율도높고, 침윤성선암을보이는경우가많아생존율이수술적절제이후에도낮다고보고되어있다. 3 담관의관내유두종양은췌관내점액유두종양보다그수가매우적어, 국내에서도 2000년보고이래로몇몇의보고가있었으나, 4,7,8 이번증례와같이동반된감염으로인한심한화농성담도염으로인해간농양으로오인된종괴형담도확장의경우는보고된바가없다. 과분비된점액은담관의심한확장이일으키기도하며, 초음파검사에서무에코로관찰되나, 에코의초점으로관찰될수있다. 또한내시경역행성담췌관조영술에서점액은작고, 다발성, 무정형의충만결손으로나타나며, 점액분비는췌장의유두상점액종양보다담관의유두상점액종양에서빈번하게관찰되는것으로나타났다. 3 복부초음파, 전산화단층촬영, 복부자기공명영상진단등의검사에서간내낭선종, 간농양등과의감별이어려울수있어담관과의연결유무가중요한데, 이런경우 MRCP 가이많은도움이될수있다. 담관의관내유두종양은과형성종, 선종, 암종으로진행하는병리기전을가지고있고, 이는전암성병변으로수술하여완전절제가가능한경우생존율을증가시켜, 수술적절제에의한제거가추천된다. 3,4,8 또한 Jang 등은 13 명의담관내유두종양환자에서수술적치료또는항암방사선요법받은환자의 1년, 3년, 5년생존율을 100%, 84.6%, 59.2% 로보고하였다. 9

37 Fig. 3. (A) Gross photo shows a dilated intrahepatic duct and partially papillary growth. (B) Gross photo shows cystic dialated ducts filled with intraluminal mucinous material, and shows partially intraluminal papillary growing lesion. Fig. 4. (A) Luminal surface of dilated duct is lined by dysplastic epithelium with papillary structure (H&E, x40). (B) Duct lining epithelium shows intraluminal papillary growth, nuclear stratification, and nuclear pleomorphism as well (H&E, x100). (C) Luminal surface of dilated duct is lined by dysplastic epithelium with papillary growth. Ductal lining epithelium shows all the feature of papillary growth, marked nuclear pleomorphism, the loss of polarity, nuclear stratification, but no invasion of basal lamina, so the features satisfy the criteria of high grade dysplasia. Intraluminal mucin and inflammatory cells are noted (H&E, x100). (D) the fibrosis and inflammation in the periphery of dilated duct are observed. Periductal abscess formations are frequently observed (H&E, x12.5)

정상윤외 7 명 38 저자들은발열을주소로내원한환자에서간농양모양을보였던고도의이형성을보인담관내유두종양 1예를경험하였기에보고한다. 요약 담관내유두종양은미세한섬유혈관중심을감싸는수많은엽상의유두상주름이발생하는것을특징으로, 종양자체나혹은종양의잔해로인한폐쇄혹은과도하게분비되는점액으로인해담관이막혀, 간내담관이확장되는다발성의종양으로, 췌관내점액유두종양과개념적으로대척되어이들사이의연관성을연구중이다. 최근발열을주소로내원한환자에서복부초음파에서간농양이의심되어, 간농양의원인을찾고자시행한추가적영상검사에서담관내유두종양이의심되어수술을시행한결과, 고도의이형성을동반한점액분비하는간내담관내유두종양으로진단되었던 1예를경험하였기에문헌고찰과함께보고한다. 색인단어 : 담관내유두종양, 간, 농양 참고문헌 1. Bosman FT, Carneiro F, Hruban RH, Theise ND. WHO classification of tumours of the digestive system. 4th ed. Lyon: International Agency for Research on Cancer 2010; 196, 222-223, 270-271 2. Lim JH, Yi CA, Lim HK, Lee WJ, Lee SJ, Kim SH. Radiological spectrum of intraductal papillary tumors of the bile ducts. Korean J Radiol 2002;3:57-63. 3. Minagawa N, Sato N, Mori Y, Tamura T, Higure A, Yamaguchi K. A comparison between intraductal papillary neoplasms of the biliary tract (BT-IPMNs) and intraductal papillary mucinous neoplasms of the pancreas (P-IPMNs) reveals distinct clinical manifestations and outcomes. Eur J Surg Oncol 2013;39:554-558. 4. Park SJ, Kwon TS, Joo SH, Kim YW, Lee SM, Hong SW. Intraductal Papillary Mucinous Neoplasm of the Bile Ducts. J Korean Surg Soc 2007;73:266-271. 5. Kim DH, Kim EK, Son BK. Synchronous Undifferentiated Carcinoma of Gallbladder in a Patient with Intrahepatic Intraductal Papillary Mucinous Neoplasia (b-ipmn). J Korean Surg Soc 2010;79:415-419. 6. Jeong JH, Park H, Moon SW, et al. A Case of Macroscopically Unvisualized Mucin-hypersecreting Biliary Papillomatosis Diagnosed by Microscopy. Korean J Gastroenterol 2009;53:206-210. 7. Chen M-F, Jan Y-Y, Chen T-C. Clinical studies of mucin-producing cholangiocellular carcinoma: a study of 22 histopathology-proven cases. Ann surg 1998;227:63-69. 8. Kim HJ, Kim MH, Lee SK, et al. Mucin-hypersecreting bile duct tumor characterized by a striking homology with an intraductal papillary mucinous tumor (IPMT) of the pancreas. Endoscopy 2000;32:389-393. 9. Jang GW, Hwang S, Lee YJ, et al. Clinicopathological features of the intraductal papillary neoplasms of the intrahepatic bile duct. Korean J Hepatobiliary Pancreat Surg 2012;16:138-141.